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358 Cards in this Set

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Antidote for Heparin over-anticoagulation
Protamine.
Mechanism of Heparin
Activates Antithrombin III
Mechanism of Warfarin
inhibits vitamin K
Antidote for Warfarin over-anticoagulation
Vitamin K
Mechanism of Enoxaparin
inhibits factor Xa
Petechiae indicate dysfunction of what?
platelets
Hemarthroses indicate disorder of what?
coag factors
Heparain Bridge is required due to a temporary hypercoagulability before Warfarin is able to inhibit Vit K dependent factors to anti-coagulate. What is the mechanism?
Protein C and S are inhibited first
What are the Vit K dependent coag factors?
II, VII, IX, X (remember there are four; II+VII=IX, and one more: one+IX=X)
Most common Hemophilia (90%).
Hemophilia A (Factor VIII defic)
Hemophilia B makes up what percent of all hemophilias?
9% (remember Factor IX deficiency = 9 percent)
Hemophilia A is deficiency in what coag factor?
VIII (remember EIGHT sounds like A)
V-Lieden is clotting disorder because factor V is resistant to what?
Activated Protein C (C+S=APC)
Hemophilia B is deficiency in what coag factor?
IX
Intrinsic Coag Cascade pathway is measured by what blood test?
PTT
Heparin elevates what coag blood test?
PTT
Warfarin elevates what coag blood test?
PT
Extrinsic Coag Cascade pathway is measured by what coag blood test?
PT
Most common inherited Bleeding Disorder (1% of the population)?
von Willebrand's Disease (vWD)
vWF normally binds what coag factor?
VIII
Hemophilia A has what form of inheritance?
X-linked Recessive
Hemophilia B has what form of inheritance?
X-linked Recessive
Why does arthropathy and joint destruction occur in untreated Hemophiliacs?
Hemosiderin deposits cause fibrosis
How to differentiate between Clotting Factor Deficiency and Clotting Factor Inhibitor.
Mixing study: mix pt blood with normal blood and test PTT: if it does not correct, likely Inhibitor present.
Mild symtoms of Hemophilia develope around what level of clotting factor levels (compared to normal)?
5% of normal
Treatment for bleeding Hemophiliac?
Cryoprecipitate
What does the half life of Factor VIII mean for treatment in Hemophilia A who is bleeding?
12 hours (if bleeding, will need cryoprecipitate BID)
What does the half life of Factor IX mean for treatment in Hemophilia B who is bleeding?
24 hours (if bleeding, will need cryoprecipitate qDay)
Mild hemophilia A can be treated with this medication.
Desmopressin (DDAVP)
Patients on Desmopression should be fluid restricted to avoid this complication.
Hyponatremia
15% of Hemophilia A patients have to stop taking replacement factor VIII. Why?
they develop neutralizing IgG antibodies to Factor VIII
von Willebrand's Disease (vWD) has what form of inheritance pattern?
Autosomal Dominant
This over the counter medication is contraindicated in vWD.
Aspirin (avoid platelet inhibitors)
Usual location of bleeding in vWD.
mucosal (e.g. nose bleeds)
How do female patients with vWD usually present?
menorrhagia
Treatment for patient with vWD who is actively bleeding.
Desmopressin (DDAVP)
What is the Ristocetin Cofactor Asay (for vWD)?
measures vWF ability to agglutinate platelets in vitro
Most common inherited Thrombophilia.
Factor V Leiden
What is the function of Activated Protein C (APC)?
inhibits Factor V (inhibits clotting cascade)
Why will vWD patients sometimes have elevated PTT?
relative VIII deficiency
Why would women with Factor V Leiden often present first to their OB/GYN doctor?
may have recurrent miscarriages
CLOTTING-SX mnemonic for acquired hypercoagulable states.
Cancer
Lupus Antigen/antiphospholipid syndrome
Obesity
Trauma
Tobacco
Immobility
Nephrotic syndrome
Gravid
Surgery
X-(tx)-Meds (e.g. OCPs)
3 Vitamins to give in treatment of Hyprehomocysteinemia.
B6 alone or with: B12 and Folate
No identifiable cause for clotting. What hypercoagulability labs to order? (7) Px-in-CALVES mnemonic
Prothrombin G0210A
C-protien
Antithrombin III
Lupus Antigen/Antiphospholipid Syndrome
V Leiden (APC resistance)
Extra Homocystein
S-protein
3 main cuases of DIC.
Delivery (obsetric stuff)
Infection (epecially septicemia)
Cancer (APML aka AML M3)
In DIC, which coag factor is elevated?
PT and PTT
In TTP, which coag factor is elevated?
neither PT or PTT is elevated
In vWD, which coag factor is elevated?
PTT
In Hemophilia, which coag factor is elevated?
PTT
In liver disease which coag factor is elevated?
PT and PTT
How to differentiate chronic DIC from Liver disease on Step 2?
D-Dimer markedly elevated in DIC
Triad of Hemolytic Uremic Syndrome (HUS).
Microangiopathic Hemolytic Anemia
Renal Failure
Low Platelets
TTP pentad
Low Platelets
Microangiopathic Hemolytic Anemia
Renal Failure
Neurologic Signs
Fever
What is HELLP syndrome?
Hemolytic anemia
Elevated Liver enzymes
Low Platelets
Key determinant of calling it TTP vs HUS on lab findings.
More Severe Renal Failure is more likely HUS
1st DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
3-6 months
2nd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
6-12 months
3rd DVT or other clotting event; how long to treat with anticoagulation (Warfarin)?
Lifetime
Thrombosis and Hemorrage occur in DIC. What is the mechanism of the clotting?
deposition of fribrin in small vessels
Thrombosis and Hemorrage occur in DIC. What is the mechanism of the bleeding?
depletion of clotting factors
Hospital inpatient developes petechiae, then starts oozing blood from IV site; high D-dimer. Diagnosis?
DIC
Petichea suggest deficiency of what?
platelets
Bleeding into boy cavities or joints suggests deficiency of what?
clotting factor/s
When to give platelets to a patient with DIC.
If 1) bleeding and 2) plts less than 20,000
How to differentiate DIC from liver disease?
VIII is not decreased in liver dx, but is depleted in DIC
Key determinant of calling it TTP vs HUS in history.
HUS associated with recent/current diarrhea; also it's most commonly called HUS in kids
HSP differential from TTP in patient with purpura and renal failure.
HSP has normal plts and NO hemolytic anemia
1st Diagnostic Step in TTP
peripheral smear
Appearance Microangiopathic Hemolytic anemia on peripherial smear?
schistocytes
How to differentiate TTP from DIC (or Liver disease)?
clotting factors are normal in TTP
3 blood labs abnormalities that indicate hemolysis.
high LDH, low Haptoglobin, high indirect bili
When to give platelets in TTP.
Never on step 2; can give if life-threatening bleeding
1st line treatment in TTP or HUS.
plasmapheresis
If patient with TTP or HUS is not responding to daily plasmapheresis, what is tx?
plasmapheresis BID and add corticosteroids
Most common reason for low platelets in women of childbearing age?
ITP
Typical age and sex of Chronic ITP patient.
20-40 and female
Typical age and sex of Acute ITP patient.
2-6 (male=female)
Presentation of Chronic ITP
Asymptomatic with incidental thrombocytopenia
Pathophysiologic mechanism of platelet destruction in ITP.
IgG antibodies formed against platelets
Presentation of Acute ITP.
abrupt hemorrhage complications following a viral illness
Prognosis: acute vs chronic ITP
acute onset ITP: spontaneous remission
insidious onset ITP: remains chronic
What is the threshold for starting treatment (steroids +/- IVIG) in patient with ITP.
if plts are less than 20,000
1st line treatment of ITP.
Steroids: Prednison or Decadron
When to try anti-D (Rh) immunoglobin in patient with ITP.
if Steroids fail to raise plts above 20-30
Steroids, IVIG, anti-D all fail to raise plts above 20 in ITP patient. Next step in management?
immunize, then splenectomy
In pregnant patients who develop auto-immune thrombocytopenia, what happens in the fetus?
also develops thrombocytopenia
2 urine abnormalities in hemolytic anemia.
Hemoglobin (once haptoglobin is all occupied)
Hemosiderin (in sloughed tubular cells)
What cell can differentiate into a macrophage?
Monocyte
What cell produces platetets?
Megakaryocyte
What type of cell is also called a plasma cell?
B-Cell
What cell is a nucleated form of a red blood cell?
reticulocyte
PMNs are given their name by what cytologic feature?
PolyMorphic nature of the Nucleus
Lymphoblast gives rise to what 2 cell types.
B and T cells
Most common cause of anemia
Iron Deficiency
Most common cause of iron deficiency
GIB
Most common cause of iron deficiency in toddlers.
insufficient dietary intake for growth
Most common cause of iron deficiency in child-bearing aged women
menses
Mechanism of iron deficiency in pregnant pt.
insufficient dietary intake for growth
1st step in approach to anemia.
MCV (micro, normo, or macrocytic)
MCV in iron deficiency.
low (microcytic)
Tests to order if macrocytic anemia.
B12 and Folate
Anemia in elderly individual. Diagnosis?
Colon Cancer until proven otherwise
Anemic patient craving things like ice and dirt. What is this called?
Pica
fingernails in iron deficiency (especially kids).
koilonychia (spoon nails)
Causes of microcytic anemia (TICS mnemonic)
Thalassemia
Iron Deficiency
Chronic Disease
Sideroblastic
Fe defic. vs Anemia of Chronic Disease: most important component of iron studies.
ACD: TIBC up
Fe defic: TIBC down
Retic count in Fe defic.
low
Ferritin is high in patient with Fe defic. Why?
acute phase reactant
Common cause of Fe Defic especially in Africa.
Hookworm causing occult bleeding
Most common cause of B12 deficiency
pernicious anemia
What causes pernicious anemia?
destruction of parietal cells reduces secrection of intrinsic factor which means reduced B12 absorption
Besides Folate or B12 deficiency, these types of drugs which interfere with DNA synthesis also can cause macrocytic anemia.
Chemotherapy meds
Most common cause of Folate Deficiency.
Poor dietary intake
What infection can result in B12 deficiency?
Tapeworm (Diphyllobothrium latum)
B12 deficiency is a known complication of this surgery.
Gastrectomy
Demyelinating disorder (subacute combined degeneration of the cord) with neuropsychiatric dysfuntion from deficiency of this vitamin.
B12
What granulocytes changes in patients with macrocytic anemia?
hypersegmented nuclei
What is the schilling test? (classic but rarely performed)
ingestion of radiolabeled cobalim both with and without intrinsic factor
What 2 tests to order in conjunction with B12 and Folate?
mehtylmalonic acid (MMA) and homocysteine levels
On follow-up, patient with B12 and Folate deficiency now has increased reticulocytes. Why?
production is no longer inhibited by B12 and Folate deficiency even though it takes longer for the anemia to correct
MCV in hemolyitic anemias.
usually normocytic
anemia after Fava Beans ingestion. Diagnosis?
G6PD deficicney
Inheritance of G6PD deficiency.
X-linked Recessive
Most common cause of hemolytic events in patients with G6PD
infection
Black soldier in Vietnam developed hemolytic anemia after taking this medication?
anti-malarial (quinine)
Defect in a membrane cytoskeleton causes RBCs to be round. Diagnosis?
Hereditary Spherocytosis
What mutation causes Sickle Cell?
point mutation in Beta Globin (glu to val)
Dark urine secondary to hemolytic anemia. Diagnosis?
paroxysmal nocturnal hemoglobinuria
Mechanical heart valves can cause this RBC abnormality.
hemolysis
Autoimmune hemolytic anemia (AIHA) can be induced from these 2 infections.
EBV or mycoplasma
Reticulocyte count in hemolytic anemia.
high
Jaundiced patient found to have normocytic anemia. Cause?
hemolysis
How to test for autoimmune hemolysis?
Coombs test
Treatment for autoimmune hemolytic anemia.
Steroids
Supplement to give after hemolytic event?
Iron
Microangiopathic Hemolitic Anemia on peripheral smear. DDX (4)?
HUS
TTP
DIC
HELLP (obstetric complication)
Maternal serum tested on test blood at 28 weeks to see if her blood is Rh positive. What type of coombs test?
Indirect
Rh + baby's blood is tested to see if antibodies from Rh - Mom are bound to his blood. What type of coombs test?
Direct
Positive Direct vs. Indirect Coombs test. Where are the antibodies located?
Direct: antibodies attached to RBCs (attacking)
Indirect: antibodies in Serum (not attacking yet)
Positive Direct vs. Indirect Coombs test. Where are the antibodies from?
Direct: from self (autoimmune); or from transfusion (from donor or from mother to baby)
Indirect: from self (potential transfusion rxn)
Typical CBC in Aplastic Anemia
pancytopenic
Absent radii, short stature, café-aulait spots. Diagnosis?
Fanconi's Anemia
Hereditary disorder which causes Aplastic Anemia.
Fanconi's Anemia
Most common cause of Mortality in Aplastic Anemia.
infection (immunocompromised)
Sickle Cell Patient with pancytopenia. What caused the Aplastic Crisis?
Parvovirus B19
CBC shows pancytopenia. Next 2 steps in work-up?
peripheral smear then BM bx
Treatment of apalstic Anemia. (3 fold)
Blood Transfusions
Stem Cell Transplantation
Treat infections aggressively
What causes the "functional" asplenia in Sickle Cell disease (SCD)?
vaso-occlusive splenic infarction
What other common cell on peripheral smear in Sickle Cell patient (besides sickled cells)?
Target cells
Why do Sickle Cell Disease (SCD) patients have DNA remnants called Howel Jolly bodies found on Peripheral Smear
Autosplenectomy
What bacteria causes most of the osteomyelitis associated with Sickle Cell Disease (SCD)?
Salmonella
What is the risk of chronic transfusion therapy (besides transfusion rxn)?
iron overload (secondary hemochromotosis)
Infectious Disease prophylaxis in Sickle Cell Disease (2)?
pneumococcal vaccine
ppx penicillin until age 10
3 most common causes of Vaso-Occlusive Crisis in SCD?
Dehydration
Infection
Cold temperatures
Goal sickle cell variant percentage for treatment of Vaso-Occlusive Crisis in SCD (via blood transfusion)?
keep sickle variant under 40%
How to prevent acute chest syndrome (e.g. pneumonia) during vaso-occlusive crisis in SCD pt? (3)
1) transfusion until sickle variant is less than 40%
2) incentive spirometry +/- O2 supplementation
3) aggressive hydration (IVF)
What surgery is common is Sickel Cell Disease (SCD)?
cholecystecomy
SCD treatment to increase fetal hemoglobin.
Hydroxyurea
SCD treatment that is contraindicated in pregnancy (teratogenic)
Hydroxyurea
Hip problem associated with SCD?
Avascular necrosis of the hip
Patients say this is the most important management of vaso-occlusive crisis (in SCD).
pain control
Classic race associated with Thalassemia on Step 2 questions.
Mediterrainian (Middle Eastern)
How to diagnose Sickle Cell Disease (2)
1) peripheral blood smear
followed by 2) hemoglobin electrophoresis
How to diagnose Thalassemia?
hemoglobin electrophoresis
Prevention of iron overload in patients who require repetative transfusions.
Deferoxamine
Thalassemia is beta or alpha based on what?
Where the defect is (i.e. beta thalassemia is a defect in beta hemaglobin)
Completely absent Beta globin genes. What type of Thalassemia?
Beta Thalassemia Major
Missing 1 of 2 Beta globin genes. What type of Thalassemia?
Beta Thalassemia Minor
No Alpha globin genes. What type of Thalassemia?
Hydrops Fetalis
1 of 4 Alpha globin genes. What type of Thalassemia?
Hemoglobin H disease
2 of 4 Alpha globin genes. What type of Thalassemia?
Alpha Thalassemia Trait
3 of 4 Alpha globin genes. What type of Thalassemia?
Silent Carrier
Microcytic anemia develops at 6 months and requires chronic transfusions. What type of thalassemia?
Beta Thalassemia Major (0 of 2 Beta genes)
Why do symptoms not show before about 6 months in Beta Thalassemia?
Hgb F still around before then
Microcytic anemia, but asymptomatic. What type of thalassemia?
Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes)
Patients die in utero. What type of thalassemia?
Hydrops Fetalis (0 of 4 Alpha genes
Severe microcytic anemia from birth and requires chronic transfusions. What type of thalassemia?
Hemoglobin H disease (1 of 4 Alpha genes)
1/3 of patients develop skeletal changes due to increased erythropoiesis. What type of thalassemia?
Hemoglobin H disease (1 of 4 Alpha genes)
Microcytic anemia, but asymptomatic. What type of thalassemia?
Beta Thalassemia Minor (1 of 2 Beta genes) OR
Alpha Thalassemia Trait (2 of 4 Alpha genes)
Person has thalassemia but does not know it because is asymptomatic and no anemia present. What type?
Silent Carrier (3 of 4 Alpha genes)
Why are dehydrated patients often pan-polycythemic?
Cells are hemoconcentrated because of decreased plasma volume
What is the level of erythropoetin in Polycythemia Vera?
low
Symptomatic treatment for polycythemia induced hypoxia from hyperviscosity syndrome.
phlebotomy
Cytoreductive Treatment of Polycythemia Vera (PCV)?
Hydroxyurea or Interferon
Polycythemia Vera patients are pro-thrombotic, and therefore should be treated with this med.
Aspirin
Prognosis of Polycythemia Vera (PCV)?
7-10 years with treatment
Clonal proliferation of a pluripotent marrow stem cell which causes erythrocytosis. Diagnosis?
Polycythemia Vera (PCV)
Polycythemia found in patient and Erythropoetin (EPO) level is high. Diagnosis?
EPO producing tumor
Why do Incan natives often have high RBC counts?
adaptation to high altitude hypoxia
Patient with polycythemia. What 2 tests to order.
EPO level (low in PCV)
Imaging (check for neoplasms)
2 most common types of blood transfusion reactions.
Nonhemolytic febrile reaction
Minor allergic reaction
1st step in treatment of a blood transfusion reaction.
stop the transfusion!
Dramatic hemoglobinuria after hemolytic transfusion reaction may lead to this.
acute tubular necrosis and renal failure
1-6 hours after transfusion, patient gets fever. Most likely cause?
Nonhemolytic febrile reaction
Most common cause of hemolytic transfusion reaction.
ABO incompatibility from clerical error
Anaphylactic reaction to blood transfusion. What immunodeficiency?
IgA deficiency (anaphylaxis to donor IgA)
Minor allergic reactions to blood transfusions have this symptom and often nothing more.
Hives (urticaria)
Transfusion reaction with immediate burning at IV site, tachycardia, fever, nausea, flushing, hypotension.
Hemolytic reaction
Treatment for hemolytic transfusion reaction.
1) stop transfusion
2) IVF
3) increase renal blood flow (pressors and diuretics)
Treatment of nonhemolytic febrile transfusion reaction?
acetominophen
Treatment of minor allergic reaction to transfusion
antihistamines (diphenhydramine)
Abnormality of heme productions that can cause photodermatitis, neuropsychiatric complaints, or colicky abdominal pain. Diagnosis?
Porphyria
Pink Urine. Diagnosis
Porphyria
Hemolytic Anemia precipitated by fasting. Diagnosis?
Erythropoietic Porphyria
Brown urine after a party where pt consumed alcohol and barbiturates. Diagnosis.
Erythropoietic Porphyria
Treatment for a mild attack of porphyria?
high doses of glucose
Treatment for severe attak of porphyria (and mechanism)?
IV hematin (negative feedback to heme synthetic pathway)
Most common porphyria. Presents with skin erythema and blisters (photodermatitis) and no abdominal pain.
Porphyria cutanea tarda
This blood disorder commonly presents with neuropsych complaints. Often mistaken for Guillain Barre.
Porphyria
Typical age in Acute Lymphocytic Leukemia (ALL).
under 15; "L" for Little people
Most common childhood malignancy.
Acute Lymphocytic Leukemia (ALL); "L" for Little people
Typical age in Acute Myelogenous Leukemia (AML).
usually under 40; "M" for middle
The 4 oncologic emergencies.
Hypercalcemia
Spinal Cord Compresion
Superior Vena Cava Syndrome
Leukostasis
ALL presentation.
anemia (pallor, fatigue)
thrombocytopenia (petichiae, purpura, bleeding)
medullary expansion (bone pain)
Look for this on abdominal exam in patients with Acute Leukemias.
hepatosplenomegally
Diagnostic test for Acute Leukemias
BM aspiration or peripheral blood if blassts are presnt
Marrow aspirate criteria which is diagnostic for Leukemia.
more than 20% blast cells
Auer rod. What specific type of AML?
APML (Type M3 AML)
Which has scant cytoplasm: Lymphoblast or Myeloblast?
Lymphoblast has less cytoplasm
Describe CBC in acute leukemias (AML and ALL)
anemia
thrombocytopenia
leukocytosis (non-functional); with neutropenia
FAB sytem subtypes of ALL?
L1, L2, L3
FAB sytem subtypes of AML?
M0 to M7
Which FAB system subtype of AML has the best prognosis? Why?
M3, because it responds to all-trans-retinoic acid (ATRA) therapy
Mainstay treatment for acute leukemias (AML, ALL).
chemotherapy
symptomatic treatment for acute leukemias (AML, ALL)
transfusions, antibiotics
Treatment for AML or ALL refractory to chemo.
BM transplant
Why give allopurinol to ALL and AML patients?
during chemo to prevent tumor lysis syndrome from hyperuricemia and renal insufficiency
Risk of this complication in ALL or AML when WBC count is higher than 100,000.
Leukostasis
Treatment for Leukostasis (blasts occluding the microciruclation causing lung, brain, and bleeding problems).
Hydroxyuria +/- leukapharesis to rapidly decrease WBC count
Which FAB system subtype of ALL has a poor prognosis due to the association with Burkitt's Lymphoma?
L3
What percent of ALL patients undergo complete remission with Chemo?
85%
The most common type of leukemia.
Chronic Lymphocytic Leukemia (CLL)
Almost all cases of Chronic Lymphocytic Leukemia (CLL) are clonal expansions of this type of cell.
Well differentiated B-Lymphocytes
Typical age and sex of CLL patients.
over 60 y/o and Male (2:1); "CL" for SEEniLe (old people)
Typical age of CML patients
30-60; "M" for middle
Flow cytometry scatter plot shows what antigens in CLL cells?
CD5 (T-cell antigen) and CD20 (B-Cell antigen)
List the 4 poor prognostic symptoms/signs in the Rai Staging of CLL from bad to worst (LSAT mnemonic)
0: Lymphocytosis (no treatment at this stage)
I: Lymphadenopathy (ok prognosis; needs chemo)
II: Splenomegaly
III: Anemia
IV: Thrombocytopenia (worst prognosis)
When to start Chemo treatment in CLL
When symptomatic (recurrent infections or lymphadenopathy
CLL patient now with high LDH. Dx?
Autoimmune Hemolytic Anemia
Why do CLL patients sometimes get BM bx even though staging is based on sx?
BM bx can help assess fi responding to therapy
What other immunodeficiency do CLL patients often develop (besides the Leukocytosis with functional Leukopenia)?
Hypogammaglobulinemia
Why do lymph nodes and spleen get enlarged with CLL?
lymphocyte accumulation
Why do CLL patients often get anemia and thrombocytopenia (poorer prognosis)?
lymphocyte accumulation in the Bone Marrow
Is CLL curable?
no, but disease-free intervals can be attained
What is the name of the BCR-ABL translocation in CML?
Philadelphia Chromosome t(9,22)
Most common presentation of CML.
asymptomatic: usually found in work-up of
incidental anemia +/- leukocytosis
on routine CBC check
Symptoms of progressing CML.
typical constitutional cancer sx: weight loss, anorexia
3 phases of CML and time in them
1) Chronic: usually asymptomatic; 5 years, longer if on treatment
2) Accelerated: the transition (days); abrupt increase in basophils and decrease in plts
3) Blast: looks like acute leukemia; survival is 3-6 months
Selective inhibitor of BCR-ABL tyrosine kinase.
Imatinib (Gleevec)
Treatment for CML in blast stage (last stage).
can try stem cell transplant or chemo; can try a clinical trial or whatever
What shows on Peripheral Smear with CML?
Leukocytosis, granulocytes in all stages of maturation
Typical WBC count in patient with CML.
Very high (100,000-500,000)
Typical age and sex in Hairy Cell Leukemia a rare malignancy (2% of adult leukemias).
HCL is similar to CLL in symptoms and presentation: Older than 55 and Male (5:1); "CL" for SEEniLe
Cell type in Hair Cell Leukemia (HCL).
Well differentiated B-Lymphocytes (again just like CLL)
CBC in CLL.
diff shows Lymphocytosis (Lymphs over 5000)
What Leukemia is a misonomer?
HCL (usually leukopenic)
1st diagnostic step in HCL (sensitivity lacks).
peripheral blood smear: see Hairy Cells on smear in 85% of HCL patients
Confirmatory test in HCL.
BM bx with TRAP staining
What percent of HCL patients have a benign course and never require therapy?
10%
When to initiate treatment in HCL.
symptomatic due to cytopenias
Treatment in HCL.
nucleoside anologs (e.g. cladribine)
Diagnostic test necessary for Lymphomas.
Excisional lymph node biopsy
90% of lymphomas are clonal expansions of this type of cell.
B-Cell
Another name for Chronic Lymphocytic Leukemia.
Small Lymphocytic Leukemia (SLL)
Lymphoma grades I-IV (Ann Arbor classification)
I - one lymph node
II - lymph nodes on one side of the diaphragm
III - lymph nodes on both sides of the diaphragm
IV - Marrow involved
4 parts of tumor lysis syndrome (PUCK)
HyperPhosphatemia
HyperUricemia
HypoCalcemia
HyperKalemia
Most common (40%) Lymphoma in childhood (adolescent males)
T-Cell Precursor Leukemia/Lymphoma
Follicular Lymphoma translocation
t(14,18)
Diffuse Large B-Cell Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma
high grade, aggressive Non-Hodgkins type; so it gets aggressive therapy
MALT gastric lymphoma (5% of lymphomas) is caused by what?
H. Pylori
Follicular Lymphoma; rule about treatment for Non-Hodgkin's Lymphoma
Low grade, indolent Non-Hodgkins type; so it gets palliative treatment
Treatment for Lymphomas (when symptomatic).
Radiation and Chemo
Lymphoma Chemo regimine (CHOP).
Cyclophosphamide (Cytoxan)
Adriamycin (hydroxydaunorubicin)
Oncovin (vincristine)
Prednisone
Pancytopenia and Lymphadenopathy. Next steps in work-up?
Lymph-node excisional biopsy
Indolent Lymphomas present how?
painless lymphadenopathy
cytopenia
hepatosplenomegaly
Aggressive Lymphomas present how?
fever, night sweats, weight loss
bulky adenopathy
extranodal masses (GI, GU, skin, thryoid, CNS)
EBV, HTLV-1, HCV have been known to introduce exogenous genes. They are dubbed "oncogenic viruses" for causing this type of cancers.
Lymphoma
Hodgkin's Disease (HD) is a monoclonal expansion of this type of cell.
B-Cell
Usual stage of presentation in Hodgkin's Disease.
Stage 2 (usually above the diaphragm)
ABVD chemo regimen option for Hodgkin's Disease (HD).
Adriamycin
Bleomycin
Vinblastine
Dacarbazine
MOPP chemo regimen option for Hodgkin's Disease (HD).
Mechlorethamine
Oncovin (vincristine)
Procarbazein
Prednisone
Owls-eye cells: Reed-Sternberg Cells (giant abnormal B cells with bilobar nuclei and huge eosinophilic nucleoli). Diagnosis?
Hodgkin's Disease
Treatment for Hodgkin's Disease.
Chemo and Radiation
As survival increases for Hodgkin's Lymphoma, this complication of radiation has become a popular concern.
radiation induced secondary neoplasms
A clonal proliferation of malignant plasma cells; diagnosis?
Multiple Myeloma
Bone pain at rest. Suspect what?
Malignancy
Why isn't presence of monoclonal M proteins sufficient to diagnose Multiple Myeloma.
MGUS is common; also other lymphoproliferative disorders can express M protein
Why are Multiple Myeloma patients prone to infection?
overproduction of M protein causes failure to produce other antibodies in sufficient quantity
Anemia and bone pain. Suspect what?
Multiple Myeloma
Triad of diagnosis for Multiple Myeloma
over 10% plasma cells in BM (BM bx)
M-protein spike (SPEP or UPEP)
evidence of lytic bone lesions (XRs)
Why hypercalcemia in Multiple Myeloma
lytic bone processes (osteoclastic activity)
Multiple Myeloma treatment
prednisone and melphalan etc. (chemo)
Multiple Myeloma treatment if refractory to initial chemo
Support a more intensive chemo dose with autologous stem cell transplantation
Why monitor Multiple Myeloma and MGUS patients with CBC and BMP? (3)
checks for specific end-organ damage (anemia, hypercalcemia, renal insufficiency)
A LymphoPlasmocytic Lymphoma
Waldenstrom's Macroglobulinemia (a NHL)
Presentation of Waldenstrom's Macroglobulinemia: insidious or aggressive lymphoma?
insidious sx of non-hodgkin's lymphoma
Considered a precursor to Multiple Myeloma
Monoclonal Gammopathy of Undetermined Significance (MGUS)
DDX of Multiple Myeloma: elevated M protein with Raynaud's phenomenon and neuro sx.
Waldenstrom's Macroglobulinemia (a NHL)
Work-up for Multiple Myeloma with bone marrow biopsy showed Dutcher bodies (PAS+IgM deposits around the nucleus). Diagnosis?
Waldenstrom's Macroglobulinemia (a NHL)
Tissue is infiltrated by IgM and neoplastic plasma cells. Diagnosis?
Waldenstrom's Macroglobulinemia (a NHL)
Treatment of Waldenstrom's Macroglobulinemia? (2)
plasmapheresis (for high IgM)
chemo (for underlying lymphoma)
Extracellular deposits of protein fibrils. Diagnosis?
Amyloidosis
3 major organs effected by Systemic Amyloidosis.
Kidney
Heart
Liver
Amyloid deposits limited to the brain. Diagnosis?
Alzheimer's disease
Congo red staining. Diagnosis?
Amyloidosis
Amyloid L (AL), overproduction of Monoclonal Light Chain fragments, is associated with what 2 diseases?
Multiple Myeloma and Waldenstrom's Macroglobulinemia (a NHL)
Multiple Myeloma and Waldenstrom's Macroglobulinemia: which is the most common cause of M-protein monoclonal overproduction?
Multiple Myeloma is 30x as common (WM is rare)
Treatment for AL, a primary Amyloidosis.
Chemotherapy (experimental) to reduce protein burden
Treatment for Dialysis related deposition of Beta microglobulin (Amyloidosis).
Can try kidney transplant
Treatment for Heritable Amyloidosis (e.g. prealbumin deposits).
Can try liver transplant
Chronic inflammation (e.g. RA) stimulates repetitive production of an acute phase reactant AA. What is AA?
a type of Amyloidosis (amyloid A)
Neutropenia and hypothermia. Suspect what?
Fungemia
Neutropenia and fever. Treatment?
Broad-spectrum antibiotics immediately
Neutropenia with anemia or thrombocytopenia; next step in diagnosis
BM bx
Neutropenia with oculocutaneous albinism. Diagnosis?
Chediak-Higashi syndrome
Patient with isolated neutropenia has no known drug exposures, no family history; next step in diagnosis?
BM bx
Following neutropenia in Chemo patient. What treatment can shorten duration of neutropenia?
G-CSF (granulocyte colony stimulating factor)
Acute neutropenia. Usual complication.
Sepsis
Chronic neutropenia (e.g. collagen vascular disease). Usual complications.
Mouthish: stomatitis, gingivitis, sinusitis
Anusish: peri-rectal infection
Causes of Secondary Eosinophilia (NAACP)
Neoplasm
Allergies
Asthma
Collagen vascular disease
Parasites
What 2 parts of the WBC diff adds up to Neutrophil Count?
Segs and Bands
What is the definition of Eosinophilia?
over 350 (Eos% x WBC count)
Definition of Neutropenia
under 1500 (Segs% + Bands% x WBC count)
Hematuria and Eosinophilia found after returning from Afria. Diagnosis?
likely schistosomyasis
Patient with atopic dermatitis found with incidental eosinophilia. Should be questioned about what sx?
Asthma sx (sob, etc.)
Why take a diet history in patient with Eosinophilia?
find possible allergens
Coccidioidomycosis or helminth infection will cause eosinophilia in 2 locations. Where?
Blood and CSF
Treatment of Hypereosinophilic Syndrome (HES), a primary eosinophilia.
Corticosteroid and cytotoxic agents
What is an autologous transplant?
transplant from donor to self (e.g. stem cell)
In Allogenic (non-twin) transplants, what needs to be matched?
ABO and HLA
Timing of the 3 types of transplant rejection.
Hyperacute: minutes
Actue: days to months
Chronic: months to years
Pathomechanism of the 3 types of transplant rejection.
Hyperacute: preformed antibodies
Actue: T-cell mediated
Chronic: chronic immune rxn causing fibrosis
Treatment for hyperacute transplant rejection (within minutes).
Cytotoxic agents
1st line treatment to prevent/treat acute (T-cell mediated) transplant rejection (within months).
Short course of high-dose corticosteroids
What is the mechanism of Graft Versus Host Disease (GVHD)?
donated T-cells attack host tissues
Treatment for GVHD
high dose corticosteroids
Why is it better for Leukemic patients getting bone marrow transplants to get it allogenically instead of autologously?
Graft versus host-Leukemia effect where donated T-cells react against patient's leukemic cells
5 weeks after transplant, patient develops skin changes, cholestatic liver dysfunction, obstructive lung disease, or GI problems. Suspect what?
Graft Versus Host Disease (GVHD)
Triple therapy if short steroid course fails to manage Acute (T-cell mediated) transplant rejection.
Corticosteroid
Tacrolimus
Azothioprine
Associated Neoplasm: Down Syndrome
ALL
Associated Neoplasm: Xeroderma Pigmentosum
Squamous cell and basal cell carcinomas of the skin
Associated Neoplasm: Chronic Atrophic Gastritis
Gastric adenocarcinoma
Associated Neoplasm: Postsurgical gastric remnants
Gastric adenocarcinoma
Associated Neoplasm: Pernicous Anemia
Gastric adenocarcinoma
Associated Neoplasm: Tuberous Sclerosis
Astrocytoma and cardiac rhabdomyoma
Associated Neoplasm: Actinic Keratosis
Squamous Cell carcinoma of the skin
Associated Neoplasm: Barrett's Esophagus (GERD)
Adenocarcinoma of the Esophagus
Associated Neoplasm: Plummer Vinson Syndrome
Squamous Cell Carcinoma of the Esophagus
Associated Neoplasm: Cirrhosis
Hepatocellular Carcinoma
Associated Neoplasm: HBV
Hepatocellular Carcinoma
Associated Neoplasm: HCV
Hepatocellular Carcinoma
Associated Neoplasm: Ulcerative Colitis
Colonic Adenocarcinomar
Associated Neoplasm: Paget's Disease of the Bone
Secondary osteosarcom and fibrosarcoma
Associated Neoplasm: Immunodeficiency
Lymphoma
Associated Neoplasm: AIDS
Aggressive Lymphoma (NHL) and Kaposi's Sarcoma
Associated Neoplasm: Myasthenia Gravis
Thymomas
Associated Neoplasm: Acanthosis Nigricans
visceral (stomach, lung, breast, uterus)
Associated Neoplasm: Nevus
Melanoma